Successful use of daily intravenous infusion of C1 esterase inhibitor concentrate in the treatment of a hereditary angioedema patient with ascites, hypovolemic shock, sepsis, renal and respiratory failure

Abstract

Hereditary angioedema (HAE) is a rare autosomal dominant disease most commonly associated with defects in C1 esterase inhibitor (C1-INH). HAE manifests as recurrent episodes of edema in various body locations. Atypical symptoms, such as ascites, acute respiratory distress syndrome, and hypovolemic shock, have also been reported. Management of HAE conventionally involves the treatment of acute attacks, as well as short- and long-term prophylaxis. Since attacks can be triggered by several factors, including stress and physical trauma, prophylactic therapy is recommended for patients undergoing surgery. Human plasma-derived C1-INH (pdC1-INH) concentrate is indicated for the treatment of both acute HAE attacks and pre-procedure prevention of HAE episodes in patients undergoing medical, dental, or surgical procedures. We report the first case of a patient with HAE who experienced an abdominal attack precipitated by a retroperitoneal bleed while being converted from warfarin to heparin in preparation for surgery. Subsequently, the patient had a protracted course in hospital with other complications, which included hypovolemic shock, ascites, severe sepsis from nosocomial pneumonia, renal and respiratory failure. Despite intensive interventions, the patient remained in a critical state for months; however, after a trial of daily intravenous infusion of pdC1-INH concentrate (Berinert®, CSL Behring GmbH, Marburg, Germany), clinical status improved, particularly renal function. Therefore, pdC1-INH concentrate may be an effective treatment option to consider for critically-ill patients with HAE.

Multifunctional roles of leader protein of foot-and-mouth disease viruses in suppressing host antiviral responses

“…The interaction of the two eIF4G proteins with IRES is an essential event for promoting IRES activity. Therefore, viral RNA translation is unaffected [ 48 , 50 ].…”

Review: Immunology of Sinusitis, Trauma, Asthma, and Sepsis

“…3 genetic analysis of C1-INH and other proteins involved in HAE. 59 Pham et al 60 recently reported on successful use of daily intravenous infusion of C1 esterase inhibitor concentrate in the treatment of a patient with HAE and ascites, hypovolemic shock, sepsis, and renal and respiratory failure. Due to the potential severity of attacks, allergists and otolaryngologists must be knowledgeable about the recognition and treatment of laryngeal angioedema.…”

Capillary leak syndrome and disseminated intravascular coagulation after kidney transplantation in a patient with hereditary angioedema - A case report -

“…Also, C1-INH concentrate is generally well-tolerated in the treatment of CLS after kidney transplantation caused by the preventive treatment of antibody-mediated rejection [ 11 ]. Daily intravenous infusions of C1-INH concentrate also showed effectiveness in patients with HAE accompanied by ascites, hypovolemic shock, and renal and respiratory failure who did not tolerate conventional ICU treatment [ 5 ]. Moreover, C1-INH concentrate is considered a multifunctional regulator of the cascade systems, which were recently shown to improve graft function by inhibiting complement activation and reducing the postoperative inflammation [ 10 ].…”

“…Thus, patients are recommended to avoid general anesthesia with endotracheal intubation, or careful prophylactic therapies before surgery are required if surgery with general anesthesia is unavoidable. Besides the well-known clinical presentations, HAE may also produce hypovolemic shock due to the tissue leakage of fluids [ 4 ] and may lead to potentially life-threatening conditions such as capillary leak syndrome (CLS) and disseminated intravascular coagulation (DIC) [ 5 , 6 ]. Here, we report a case of suspected CLS and DIC after kidney transplantation in a patient with HAE.…”